Nephrotic syndrome

Nephrotic syndrome is a complex of symptoms that occurs when kidney failure and involves a pronounced proteinuria, swelling, disturbances in the metabolism of proteins and lipids. However, it is not always pathology is accompanied by secondary and primary disorders of the kidneys, sometimes she acts as an independent nosological form.

Term used since 1949, replacing notions such as nephrosis or lipoid nephrosis and introduced into the modern nomenclature of who. Statistics indicate that this syndrome is found among all diseases of the kidneys about 20% of cases, other data indicate that 8-30% of cases. This files most often diagnosed in children ages 2 to 5 years, at least adults, whose average age from 20 to 40 years. But in medicine, describes the case of development of a syndrome in the elderly and in newborns. The frequency of its occurrence depends on ethological factor that caused it. Women suffer more often than men in the case when pathology develops on the background of rheumatoid arthritis and systemic lupus erythematosus.

The content of the article:

• Causes of nephrotic syndrome
• Symptoms of nephrotic syndrome
• Forms of nephrotic syndrome
• Complications of nephrotic syndrome
• Diagnosis of nephrotic syndrome
• Treatment of nephrotic syndrome
• Prevention and prognosis of nephrotic syndrome

Causes of nephrotic syndrome

Causes of nephrotic syndrome various, there are both primary and secondary pathology.

Causes of primary nephrotic syndrome:

• The most common kidney diseasecaused by this syndrome is glomerulonephritis, chronic and acute. Thus nephrotic syndrome develops in 70-80% of cases.

• Other independent kidney disease, against which there is nephrotic syndrome are: primary amyloidosis, nephropathy during pregnancy, and renal cell cancer.

Causes of secondary nephrotic syndrome:

• Diabetes.
• Infection: syphilis, tuberculosis, malaria.
• Connective tissue disease systemic issues: scleroderma, lupus, rheumatoid arthritis.
• Diseases of allergic nature.
• The periarteritis.
• Hemorrhagic vasculitis.
• Periodic disease.
• Septic endocarditis with a prolonged duration.
• Hodgkin's disease.
• Effects on the body of toxic substances, including heavy metals, poisons, from the bite of bees and snakes, etc.
• Cancer of other organs.
• Thrombosis of the inferior Vena cava, renal veins.
• Taking certain medicines.

Idiopathic variant of the disease development (when the cause cannot be established) most often develops in childhood.

Turning to the pathogenesis of the syndrome, it can be noted that the most common is considered the immunological theory of development.

This confirms several facts, including:

• Experiments on animals showed that the introduction of nephrotoxic serum on the background of absolute health were the development of nephrotic syndrome.
• Often pathology develops in people who have allergic reactions to pollen, hypersensitivity to different drugs.
• Except in patients with this syndrome often have autoimmune diseases.
• Immunodepressantnoe treatment gives a positive effect.

Immune complexes, which can have a damaging effect on the basal membrane of the glomeruli, formed by contact of blood antibodies antigen-foreign (viruses, allergens, bacteria, pollen, etc.) and internal antigens (DNA, proteins, tumors, cryoglobulin, etc.). Sometimes, the immune complexes are formed as a result of development of antibodies to the substance which is reproduced by the basal membranes of kidneys. Whatever the origin of immune complexes, the degree of renal damage will depend on what their concentration inside the body and how long they are on the organs affected.

Another negative effect of immune complexes is their ability to activate the inflammatory response at the cellular level. The result is the release of histamine, serotonin, enzymes. This leads to the fact that the permeability of the basal membrane becomes higher, disturbed microcirculation in the capillaries of the kidneys, formed intravascular coagulation.

Upon microscopical examination it is seen that buds have increased in size, their surface remains smooth and flat, the cortical substance is a pale gray color, and the brain – red.

Histology and optical microscopy reveals pathological changes of fatty and hyaline degeneration of the kidneys, focal proliferation of the endothelium of the organs, protein dystrophy of the departmentsthe renal tubules. If the disease has a severe course, it is possible to visualize atrophy and necrosis of the epithelium.

Symptoms of nephrotic syndrome

Symptoms of nephrotic syndrome are typical and monotonous, regardless of what has provoked the development of pathological process.

They include:

• Massive proteinuria. Is expressed in a large amount of protein (mainly albumin) in the urine. This is the main symptom of this disease, but it is not the only symptom of the disease.
• In the blood serum increases the level of cholesterol, triglycerides, with a reduction in the amount of phospholipids. As a result, the patient develops hyperlipidemia. The amount of lipids is so high that is able to paint the serum of the blood in milky white color. In the end, only one appearance of blood can be judged from the presence of hyperlipidemia. It is supposed that the increase of lipids in the blood due to their increased production in the liver with the delay in the blood vessels due to high molecular weight. Affects also metabolism of the kidneys, the drop in the level of albumin in the blood, etc.
• There is a jump of cholesterol in the blood. Sometimes it can reach 26 mmol/l and even more. But most of the growth of cholesterol moderate and does not exceed the level of 10.4 mmol/L.
• Swelling. They can be of varying severity, but are present in all patients, without exception. The swelling is sometimes very strong, to the extent that they limit the patient's mobility, becoming an obstacle to the execution of labour duties.
• The patient has General weakness, fatigue. The weakness tends to increase, especially in the case when nephrotic syndrome is present for a long time.

• Suffering appetite, increases the need for fluid, since thirst and dry mouth are present on an ongoing basis.

• The amount of urine becomes less.
• Nausea, vomiting, flatulence, abdominal pain and diarrhea is rare. Mostly these symptoms indicate a pronounced ascites.
• Characteristic headaches, dragging sensations in the lumbar region.
• During the months-long course of the disease develop paresthesia, convulsions on the background of potassium loss, pain in the muscles.
• Hydropericardium indicates shortness of breath,which disturbs the patient even during the rest, not just physical activity.
• Patients, as a rule, slow-moving, sometimes completely adynamic.
• Pale skin, the body temperature can stay at a normal level, and may be less than normal. In this regard, the skin feels cold and dry. Possible flaking, brittle nails, hair loss.
• Tachycardia develops on the background of heart failure or anemia.
• Blood pressure either reduced or normal.
• The language reveals a dense plaque, the abdomen increased in size.
• Overall metabolism is broken, as suffering from thyroid function.
• The decrease in urine output is also a constant companion of pathology. In this day the patient selects not more than one litre of urine, and sometimes of 400-600 ml. of Visible blood in the urine, usually undetectable, but it is detected by microscopic examination.
• Another clinical sign of the syndrome is hypercoagulability of blood.

Symptoms of nephrotic syndrome can grow slowly and gradually, but sometimes rapidly, which often occurs in acute glomerulonephritis.

In addition, distinguish between pure and mixed syndrome. The difference lies in the absence or presence of hypertension and hematuria.

Forms of nephrotic syndrome

Also noteworthy are the three forms of the syndrome, including:

• Recurrent syndrome. For this form of disease is characterized by frequent change of exacerbations of syndrome of remission. While remission could be achieved through drug therapy, or it happens spontaneously. However, spontaneous remission occurs rarely and primarily in children. The share of recurrent nephrotic syndrome accounts for up to 20% of all cases of the disease. Remission can be quite long in duration and sometimes reach 10 years.
• Persistent syndrome. This form of the disease is the most common and occurs in 50% of cases. For syndrome sluggish, slow, but always progressive. Stable remission was not achieved even with resistant therapy and after about 8-10 years the patient develops renal failure.

• A progressive syndrome. This form of the disease is characterized by the fact that nephrotic syndrome develops quickly and for1-3 years can lead to chronic renal failure.

Complications of nephrotic syndrome

Complications of nephrotic syndrome can be associated with the syndrome, and to provoke drugs used for its treatment.

Among the complications are:

• Infection: pneumonia, peritonitis, boils, pleurisy, erysipelas, etc. the Most severe complication is considered to be pneumococcal peritonitis. Untimely rendered antibacterial therapy can lead to death.
• Phlebothrombosis.
• Nephrotic crisis – another rare but serious complication of nephrotic syndrome. Accompanied by an increase in body temperature, abdominal pain, cutaneous erythema, preceded by vomiting, nausea, loss of appetite. Nephrotic crisis are often accompanied by rapidly progressive nephritic shock with a pronounced drop in blood pressure.
• Pulmonary embolism.
• Thrombosis of renal arteries leading to infarction of the kidney.

• A brain stem stroke.

• There are data concerning what is nephrotic syndrome increases the risk of cardiac ischemia and myocardial infarction.
• Complications from the medication for the treatment of nephrotic syndrome are expressed in allergic reactions and in the formation of ulcers of the stomach and intestines perforated ulcers, diabetes, drug psychosis, etc.

Virtually all of these complications are life-threatening for the patient.

Diagnosis of nephrotic syndrome

Clinical and laboratory research is the leading method of diagnosis of nephrotic syndrome. However, physical and instrumental methods of examination are mandatory. The doctor during examination of the patient visualizes coated tongue, swelling, dry and cool skin, as well as other visual signs of the syndrome.

In addition to the medical examination, you can assign the following types of diagnostics:

• Urinalysis will reveal the increase in the relative density, cylindruria, leukocyturia, cholesterol in the sediment. Proteinuria in the urine, typically expressed much.
• Blood work will indicate an increase in sedimentation rate, eosinophilia, increase of platelets, the declinehemoglobin and red blood cells.
• Coagulation necessary for the evaluation of blood clotting.
• Biochemical analysis of blood reveals increased levels of cholesterol, albumin, proteinaemia.
• To assess the degree of destruction of the tissues of the kidneys is necessary to conduct ultrasound with Doppler ultrasound of renal vessels, neprezentare.
• It is equally important to determine the cause of nephrotic syndrome, which requires in-depth immunological examination, possible biopsy of the kidney, rectum, gums, performing angiographic studies.

Treatment of nephrotic syndrome

Treatment of nephrotic syndrome is performed in a hospital under the control of a nephrologist.

General recommendations to physicians practicing in urology departments, are reduced to the following points:

• Compliance with salt-free diet with restriction of fluid intake and selection depending on the age of the patient amounts of protein.
• Infusion therapy with the use of Reopoligliukina, Albumin, etc.
• Receiving cytotoxic drugs.
• Diuretics.
• The immunosuppressive treatment.
• Anti-bacterial treatment.

Diuretics are important in the treatment of kidney disease. However, their intake should be strictly controlled by specialists, otherwise you may develop serious health problems. These include: metabolic acidosis, hypokalemia, leaching of sodium from the body, reducing blood volume. As larger doses of diuretics in patients with renal insufficiency or when gipoalbuminemii often complicated by shock, which is difficult to be adjusted, the treated patients should be cautious. The shorter the period diuretics, the better. Their re-appointment, it is advisable to assign only with an increase in edema and in reducing the number of detachable urine.

To relieve the patient from swelling, recommend receiving Furosemide or intravenous or oral. This is a very powerful decongestant, but its effect is short-lived.

To relieve swelling can be assigned to Ethacrynic acid. Less pronounced effect has Hydrochlorothiazide. Its effect will be noticeable after 2 hours after the initial appointment.

Another important element in the struggle with edema are diuretics that allows you to retain potassium in the body. It's such drugs as Amiloride, Triamterene, Aldactone, Verospiron. Verospiron special efficiency issues, combined with Furosemidom.

If the swelling is due to amyloidosis, corrected with diuretics, they lend themselves with difficulty.

Corticosteroids, such as Prednisone and Methylprednisolone is the treatment of choice for the use of immunosuppressive treatment of nephrotic syndrome. These drugs have a direct effect on the immune complexes, inhibiting their susceptibility to inflammatory mediators, reducing their production.

Prescribe corticosteroids in three ways:

1. Oral supplementation with the issuance of the maximum dose in the morning and subsequent reduction of the dose in 2-4 doses. The total dose calculates taking into account the work of the adrenal cortex. The treatment is carried out at the start of therapy.

2. Alternative taking Prednisone is used as maintenance therapy. The daily dose offered to the patient at intervals of one day. This allows you to maintain the achieved effect and to reduce severity of side effects from taking corticosteroids. Also to an alternative mode of taking Prednisone include the scheme of distribution of the drug to the patient daily for 3 days, then make a break in 3 or 4 days. As for the effect, these regimes, as a rule, differences in this respect have not.

3. The latest version of the use of glucocorticoids is the implementation of pulse therapy. To do this, in the blood is very high concentration of the hormone, as it is introduced intravenously drop by drop for 20 to 40 minutes. Putting an IV in it once, not more often than once in 48 hours. The number of sets and the exact dosage must calculate doctor.

Of course, you need to remember about the abundance of side effects that give the glucocorticoids, among them: insomnia, edema, obesity, myopathy, cataract. To cancel preparations to be very careful, as there is a risk of acute adrenal insufficiency.

With regard to treatment with cytotoxic drugs, are used for this purpose such drugs as Cyclophosphamide (Cytoxan, Cyclophosphamide), and Chlorambucil (Leukeran, Chlorambucil). These drugs are aimed at suppressing cell division, and they do not have selective ability to affect absolutely all dividing cells. Activation of the drug occurs in the liver.

Cyclophosphamide treated by the method of pulse therapy, injecting it intravenously, on a background of pulse therapy with corticosteroids.

Chlorambucil given orally for 2-2,5 months. The drug is prescribed mainly when recurrent form of the disease.

Antibiotic therapy is required if the syndrome develops in the background of chronic glomerulonephritis. For this purpose, often used drugs such as Cefazolin, Ampicillin, Doxycycline.

Ifthe disease has a severe course, it is necessary to conduct plazmaferez, hemosorption, intravenous Dextrans, Reopolyglucin, protein solutions.

After the patient is discharged from hospital, he was shown the supervision of the nephrologist in the polyclinic at the place of residence. Long time support is conducted pathogenetic therapy. On the possibility of its completion could decide only a doctor.

Patients recommended SANATORNO-resort treatment, for example, on the Southern coast of the Crimea. To go to a sanatorium need during remission of the disease.

As for diet, patients must adhere to therapeutic diets under the number 7. This reduces swelling, normalize metabolism and diuresis. It is strictly forbidden to include in the menu, fatty meats, foods containing salt , margarine, TRANS-fats, all legumes, chocolates, marinades and sauces. Food must be fractional, cooking methods – sparing. Water is consumed in limited quantities, its volume is calculated individually, depending on what the daily urine of the patient.

Prevention and prognosis of nephrotic syndrome

To preventive measures include resistant and early treatment of glomerulonephritis and other kidney diseases. You must carry out a thorough rehabilitation of foci of infection and in the prevention of those pathologies, which are etiological factors of this syndrome.

Medicines should be taken only those that are recommended by the attending physician. Particular caution should be exercised in relation to those funds that have nephrotoxicity or can cause an allergic reaction.

If the disease has manifested one day in the future, requires medical supervision, timely specimen collection, avoidance of sun exposure and hypothermia. With regard to employment, people with nephrotic syndrome should be restricted in terms of physical exertion and nervous overvoltage.

The prognosis for recovery depends mainly on what was the cause of the development of the syndrome, as well as how long people go without treatment, what is the patient's age and other factors. It is worth considering that complete and permanent cure to achieve can be quite rare. It is possible, as a rule, children in primary fat nephrosis.

The other groups of patients sooner or later relapse of the disease with increasing signs of violation of the kidney, sometimes with malignant hypertension. In the endthe patient develops renal failure with subsequent izotermicheskoi uremia and death of a man. So the sooner treatment is started, the more favorable the prognosis.